Abstract
Background and aims. Pemphigus is a chronic autoimmune and vesiculobollous disease that can affect
skin and different mucous membrane surfaces. Primary manifestations occur in oral cavity in almost 60% of
cases. The purpose of the present study was to evaluate the epidemiology of pemphigus in Tehran, Iran in a
20-year period.
Materials and methods. A retrospective study was conducted on the records of 1560 patients diagnosed
with different types of pemphigus in Razi Hospital of Dermatology in Tehran from March 1985 to March
2005. A questionnaire was prepared to collect information regarding age, sex, bedridden duration, pemphigus
subtype, sites of involvement, recurrence and mortality rate. Data was analyzed using chi-square test with
significant level of P < 0.05.
Results. There was a female predominance with a male to female ratio of 1:1.53. In nearly half of the patients,
only the oral mucous membranes were affected. One hundred and fifty had only skin lesions and 261
cases had both skin and oral mucosal lesions. Involvement of esophageal and vaginal mucous membranes
without skin lesions was observed in 150 patients and 298 cases had esophageal and vaginal mucosal involvement
as well as skin lesions. Pemphigus vulgaris was the most common type, with the mean age of 44.6
years. Oral mucous membrane was the most frequent location where pemphigus vulgaris was observed. 1265
patients recovered which 52.2% of them had only oral lesions. Average of bedridden duration was 2.9
months. The highest recurrence rate was seen in patients with skin lesions exclusively. There was a significant
difference between recurrences of lesions and location of involvement (P < 0.05). Thirty six patients had died
from of the disease.
Conclusion. The mean age of the disease onset in the present study was found to be a decade earlier than
the other parts of the world. Recurrence and mortality rates were lower in patients with only oral lesions and
their prognosis was better.